Summary :
Parry-Romberg syndrome (PRS) is a rare disease, charac­terized by facial progressive hemiatrophy, with subcutaneous connective and fatty tissue atrophy, sometimes affecting even adjacent muscles, bones and nerves. The involvement of inner ear is unfrequent in PRS compared to connectivitis and sclero­dermy. We report a case with a progressive SNHL in a patient with PRS. The relationship between the two is not certain. Other cases must be reported to reinforce the suspicion of relation.